SECKEL SYNDROME : CASE REPORTS

Kim, Chu-Sung; Kim, Jae-Gon; Baik, Byeong-Ju; Yang, Yeon-Mi; Jeong, Jin-Woo

Journal of the Korean Academy of Pediatric Dentistry 2010;37(1):124-129.
Published online February 28, 2010.

SECKEL SYNDROME : CASE REPORTS

Chu-Sung Kim, Jae-Gon Kim, Byeong-Ju Baik, Yeon-Mi Yang, Jin-Woo Jeong

Department of Pediatric dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University

Correspondence: Jae-Gon Kim, Tel: 063-250-2128 /, Email: pedodent@chonbuk.ac.kr

Received: 6 April 2009 • Accepted: 15 September 2009

Abstract

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine growth retardation and post-natally by dwarfism, severe microcephaly, bird-headed profile with receding chin, prominent nose, joint defects, clubfoot, sparse hair, malformation of genitourinary tract and rectum, mental retardation and hematological disorders. There is also a reduction in the number of blood cells. Dental anomalies of Seckel syndrome are crowded teeth with malocclusion, enamel hypoplasia, absence of some teeth and taurodontism. This report described the oral and maxillofacial manifestations of children associated with Seckel syndrome. Children with Seckel syndrome have several dental and skeletal irregularities. The purpose of this study was to report the dental and medical characteristics of the patient and review the literatures of Seckel syndrome.

Key Words: Seckel syndrome, Bird-headed profile, Microcephaly, Dwarfism